Dravet Syndrome: an update

Abstract

Summary Dravet syndrome is a severe epilepsy syndrome of infancy characterised by seizures of multiple types, often prolonged and particularly fever-sensitive, with onset in the first year of life, and subsequent developmental delay. This article aims to present updated data on this syndrome, for which complete and extensive clinical descriptions led to the discovery of a link with a major gene, SCN1A, on which abnormalities are found in at least 70% of the patients tested. Our review article follows and summarises the data published in a 2011 supplement issue of Epilepsia, entitled Severe Myoclonic Epilepsy –